Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed magnetic resonance images (MRIs) and clinical records of patients with infancy-onset hydrocephalus. Among 411 infants, 236 had hydrocephalus with no recognizable extrinsic cause. These children were assigned to 1 of 5 subtypes and compared on the basis of clinical… (More)

Little is known about the epilepsy that often occurs in the juvenile form of Huntington's disease (HD), but is absent from the adult-onset form. The primary aim of this study was to characterize the seizures in juvenile HD (JHD) subjects with regard to frequency, semiology, defining EEG characteristics, and response to antiepileptic agents. A multicenter,… (More)

Rhombencephalosynapsis is a midline brain malformation characterized by missing cerebellar vermis with apparent fusion of the cerebellar hemispheres. Rhombencephalosynapsis can be seen in isolation or together with other central nervous system and extra-central nervous system malformations. Gómez-López-Hernández syndrome combines rhombencephalosynapsis with… (More)

BACKGROUND
Children with hydrocephalus are at risk for epilepsy both due to their underlying condition and as a consequence of surgical treatment; however, the relative contributions of these factors remain unknown.
OBJECTIVE
The authors sought to characterize epilepsy among children with infancy-onset hydrocephalus and to examine the risks of epilepsy… (More)

OBJECTIVE Intraventricular hemorrhage (IVH) is a complication of prematurity often associated with ventricular dilation, which may resolve over time or progress to posthemorrhagic hydrocephalus (PHH). This study investigated anatomical factors that could predispose infants with IVH to PHH. METHODS The authors analyzed a cohort of premature infants diagnosed… (More)

OBJECTIVE
Hydrocephalus, a complex condition characterized by progressive accumulation of cerebrospinal fluid within the ventricular system of the brain, affects ∼ 6 in 10,000 infants and is heterogeneous in nature. Previous investigations of risk factors have not considered etiologic heterogeneity.
METHODS
We conducted a case-control study of 1748… (More)

BACKGROUND
Head-shaking stereotypies have been described in patients with neurological impairment. We noted an unusual preponderance of head shaking in patients with rhombencephalosynapsis (RES). We sought to delineate the movements further and determine whether oculomotor and vestibular testing could reveal their cause.
METHODS
Information was collected… (More)